title: Myositis-related interstitial lung disease and antisynthetase syndrome.
abstract: In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.
authors.affiliation: Interstitial Lung Disease Program, National Jewish Health, 1400 Jackson Street, Denver, CO 80206, USA. solomonj@njhealth.org
authors.forename: Joshua
authors.initials: J
authors.lastname: Solomon
authors.forename: Jeffrey J
authors.initials: JJ
authors.lastname: Swigris
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
mesh_terms: D000604:Amino Acyl-tRNA Synthetases / Q000097:blood*; D000974:Antibodies, Antinuclear / Q000097:blood; D016824:Antigens, Human Platelet / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D009220:Myositis / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy
chemical_list: D000974:Antibodies, Antinuclear; D016824:Antigens, Human Platelet; D001323:Autoantibodies; C046750:Jo-1 antibody; D000604:Amino Acyl-tRNA Synthetases